Thoracoscopic and laparoscopic plication of the hemidiaphragm is effective in the management of diaphragmatic eventration

Purpose

Diaphragmatic plication by minimally invasive surgery is thought to allow for a quick recovery and has been performed on small children. Here, we report our experience with different plication procedures to discuss how to choose among these different plication procedures in endoscopic surgery for pediatric patients with diaphragmatic eventration.

Patients and methods

We retrospectively analyzed clinical data of 27 pediatric patients (21 boys, 6 girls; median age: 12.7 months, range 2 months–3 years) admitted to our hospital between November 2008 and July 2013. Three different plication procedures were used: the “reefing the mainsail” technique (8 patients), “invaginating the diaphragmatic dome” technique (10 patients), and “pleating” technique (9 patients). Indications included ventilator dependency (7.41 %), respiratory distress (22.22 %), chronic lung lobe collapse (11.11 %), persistent atelectasis with recurrent pneumonias (18.52 %), and asymptomatic severe eventration (40.74 %).

Results

Descending distance of diaphragm after surgery ranged from 1 to 4.5 intercostal spaces (mean distance: 2.65 intercostal spaces). All patients recovered well postoperatively, except for one patient with a pneumothorax. Two patients who required respiratory support before the operation no longer required it within 7 d after surgery. Follow-up ranged from 1 to 35 months. Clinical results were satisfactory with obvious improvement in symptoms and a slight re-elevation within a distance of one intercostal space.

Conclusion

For pediatric patients with diaphragmatic eventration, different endoscopic surgeries and plication procedures all yielded satisfactory results. We believe that the choice of one procedure over the other depends only on the surgeon’s experience.     

Efficacy and safety of thoracoscopic thymectomy in the treatment of juvenile myasthenia gravis

Background

Thymectomy is a well-established treatment for generalized myasthenia gravis in adults, but predictors of long-term efficacy and the optimum timing for intervention in juvenile myasthenia remain controversial.

Purpose

To review the preoperative presentation, surgical experience, and long-term neuromuscular follow-up in patients undergoing thoracoscopic thymectomy in a single institution.

Methods

A retrospective chart review of all patients undergoing thoracoscopic thymectomy for myasthenia gravis at a tertiary referral center between 2000 and 2010 and compared to an historical cohort of trans-sternal thymectomies performed between 1970 and 1995. Age at diagnosis, presurgical medications and hospitalizations, preoperative chest imaging, presence of acetylcholinesterase antibodies, Osserman Stage, time to operative intervention, length of follow-up, DeFillipi remission scale, as well as operative and post-operative data (length of surgery, blood loss, need for chest tube, length of intubation, length of hospital stay, pathology, and complications) were recorded.

Results

Fifteen patients undergoing thoracoscopic thymectomy were identified with a mean age of 11.3 years at time of diagnosis and average treatment duration of 12.5 months prior to operative intervention. Of these patients, most presented with Osserman Stage IIB (8) or III (5) disease. Two patients presented with Osserman Stage IIa disease. There were no reported complications, no conversions to an open approach, and an average length of stay of 2.6 days. Average length of follow-up was 37.5 months, available on 13 of 15 patients. Nine of 13 (69 %) were improved (DeFillippi Class 2 or 3) at 1-month follow-up, however, the pattern of remission waxed and waned, with only 50 % reporting improvement at 1 year, 86 % at 2 years and 75 % at 3 years. Only one patient was totally off medication. No patients required postoperative hospitalization for respiratory crisis.

Conclusions

Thoracoscopic thymectomy offers a safe approach to thymic resection in children with JMG with little associated morbidity and a short hospital stay, but should not be considered curative. Rather it appears to make generalized JMG more amenable to long-term medical management.     

Congenital anomalies of the kidney and urinary tract (CAKUT) associated with Hirschsprung’s disease: a systematic review

Purpose

Congenital anomalies of the kidney and urinary tract (CAKUT), a term introduced in the late 1990s accounts for 30–50 % of cases of end-stage renal disease in children. The association of urogenital anomalies and Hirschsprung’s disease (HSCR) based on the common genetic background of enteric nervous system and human urinary tract development has been well described in the literature. However, the reported prevalence of HSCR associated with CAKUT seems to be underestimated. The aim of this systematic review was to determine the prevalence of this association and show its relationship to other syndromes.

Methods

A systematic literature search was conducted for relevant articles published between 1955 and 2014. Two online databases were searched for the terms “Hirschsprung’s disease”, “congenital anomalies of the kidney and urinary tract”, “urogenital anomalies” and “urological anomalies”. All published studies containing adequate clinical data were included. Resulting publications were reviewed for epidemiology, genetic testing, operative treatment and morbidity. Reference lists were screened for additional cases.

Results

A total of 32 articles reported 222 cases of HSCR associated with either CAKUT, “urological” or “urogenital” anomalies from 1955 to 2014. Gender was reported in a total of 68 cases, with 54 (79 %) males and 14 (21 %) females. Extent of aganglionosis was reported in 67 cases and included classical rectosigmoid disease in 38, long-segment aganglionosis in 12, total colonic aganglionosis in 12 and total intestinal aganglionosis in 5 patients. 18 articles reported 204 cases of either CAKUT, “urological” or “urogenital” anomalies in a case series of 5.693 HSCR patients, resulting in an overall prevalence of 3.6 % of this association. Within this collective of 18 studies only seven were, regardless of the date of publication compatible with CAKUT criteria introduced and published in the late 1990s. These seven studies reported a total of 72 patients with associated CAKUT among 757 HSCR patients resulting in a prevalence of 9.5 %. After introduction of the CAKUT acronym, only three studies specifically investigated the association of HSCR and CAKUT stating a prevalence of 14.3 % resulting in an almost fivefold increase compared to the reported prevalence of HSCR and associated urological and urogenital anomalies. The remaining 14 publications reported 18 single cases of HSCR patients with associated CAKUT phenotypes. Of these 18 cases, 11 (61 %) cases were associated with other syndromes or syndromatic features or reported chromosomal anomalies.

Conclusion

This review confirms that the recognition of CAKUT in HSCR patients has been underestimated in the past. The results suggest that when confronted with HSCR in a patient, a thorough urological investigation may be indicated. The high prevalence of associated syndromes in HSCR with CAKUT may further suggest a syndromic association.     

Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: results from a nationwide survey

Purpose

Intestinal neuronal dysplasia Type B (IND-B) has been proposed to be an allied disorder of Hirschsprung’s disease (ADHD). The original histological criteria included hyperganglionosis, giant ganglia, ectopic ganglion cells and an increased AChE activity in the lamina propria. The criteria for IND-B have been gradually revised. The present diagnostic criteria are [1] more than 20 % of the submucosal ganglia contain nine or more ganglion cells and [2] the patient is older than 1 year. To clarify the current status of IND-B in Japan, a nationwide retrospective cohort study was performed.

Methods

Questionnaires were sent to 161 major institutes of pediatric surgery and gastroenterology in Japan.

Results

A total of 355 cases of ADHD were collected, including 18 cases of IND-B (5 %). Based on original criteria, 13 out of 18 cases were diagnosed as IND-B. However, only four cases met the current criteria. Three of the four patients (75 %) required pull-through operation. All of the patients exhibited giant ganglia and ganglioneuromatosis-like hyperplasia of the myenteric plexus.

Conclusions

IND-B cases matching the current criteria are thought to be quite rare and they are associated with marked hyperplasia of the myenteric plexus. “True” IND-B is a rare and intractable disease.     

Prevalence of Hirschsprung’s disease in premature infants: a systematic review

Purpose

Intestinal dysmotility in preterm infants has often been attributed to immature enteric nervous system. It is frequently reported that Hirschsprung’s disease (HD) is rare in premature infants. The exact prevalence of HD in premature infants is not well documented. The classical signs of HD may often not be identified due to the complexity of symptoms of prematurity itself. This systematic review was designed to determine the prevalence and presentation of HD in premature infants.

Methods

A systematic review of the literature using the keywords “Hirschsprung’s disease”, “aganglionosis”, “congenital megacolon”, “premature” and “preterm” was performed. Resulting publications were reviewed for epidemiology and morbidity. Only infants born <37 weeks of gestation or described as preterm birth by the authors were included. Reference lists were screened for additional relevant studies.

Results

Twenty-six publications from 1964 to 2013 reported data on premature infants with HD. Out of a total number of 4,147 infants, prematurity was recorded in 257 cases, giving a prevalence rate of 6 % of preterm infants diagnosed with HD. During 1964–1999, reported prevalence of HD in premature infants ranged from 1.7 to 9.2 % (overall prevalence 5 %) and during 2000–2013 prevalence ranged from 4 to 19.4 % (overall prevalence 14 %). The prevalence of total colonic aganglionosis in premature infants was 13 % (15 out of 118 infants). Mean gestational age of preterm infants was 34.5 (±0.7) weeks and mean age at diagnosis ranged from 18.3 days to 3.9 months. Abdominal distension was observed in 80 % of preterm infants, delayed passage of meconium in 57 and 37 % of premature infants presented with bile-stained vomiting.

Conclusion

In recent years, higher prevalence of HD has been reported in premature infants compared to previous years. Hirschsprung’s disease should be considered in preterm infants presenting with features of intestinal obstruction.     

Pediatric Moyamoya Disease: Clinical Profile, Literature Review and Sixteen Year Experience from a Tertiary Care Teaching Institute

Objective

To assess the clinical spectrum and outcome of pediatric moyamoya disease (MMD) in Indian sub-continent.

Methods

The authors retrospectively analysed data of 23 patients, diagnosed with pediatric moyamoya disease from a single center during the period of 1996–2011.

Results

There were 18 boys and 5 girls. Mean age at onset of symptoms was 3.8?±?2.2 y. All patients presented with cerebral ischemic events. Recurrent stroke was the presenting feature in 12(52.2 %) patients. Twenty one patients were definitive case of moyamoya disease and two were probable case of moyamoya disease. Posterior circulation was involved in 26.1 % patients. Three patients underwent indirect surgical revascularisation procedure and rest were managed conservatively. On follow up 66.6 % patients had residual neurological deficit.

Conclusions

Heightened awareness of this entity among pediatric neurologists is required for early diagnosis of pediatric moyamoya disease.     

Postoperative intussusceptions in children and infants: a systematic review

Background

Postoperative intussusception (POI) is an unusual complication in children and infants who underwent various kinds of surgery. The early recognition was difficult for its rarity and atypical presentations. This study evaluates the clinical features of POI through a literature review.

Methods

MEDLINE database was searched for relevant articles that reported the children and infants with POI since 1990 in English-language using the key word “postoperative intussusception”. All published studies containing clinical data for POI in children and infants were included. Reference lists of retrieved articles were reviewed for additional cases. Detailed data of the included cases were extracted and analyzed.

Results

Twenty-six studies with total 127 cases of POI were included. According to the extracted data, the median age was 19 months with the male-to-female ratio 1.5:1. There were 65 operations (51.2 %) that involved gastrointestinal system, 26 cases (20.5 %) of retroperitoneal tumor resection, 12 operations (9.4 %) involved diaphragm, 8 operations (6.3 %) involved urinary system, 5 cases (3.9 %) of partial pancreatectomy, 11 cases (8.7 %) of non-abdominal operations. 75.5 % presented symptoms in the first 7 days after surgery. The prominent symptom was bilious vomiting or increased nasogastric output (87.1 % of 101 patients), following abdominal distention (74.3 %), abdominal pain (35.6 %). Six cases (5.0 %) of ileocolic POI were reduced successfully by air enema. The small bowel intussusception attributed 85.6 % of POI (95 patients). Laparotomy and manual reduction were performed in 104 cases (86.0 %). Nine patients (7.4 %) underwent intestinal resection and anastomosis.

Conclusions

POI should be suspected in pediatric surgical patients who showed signs of intestinal obstruction in the early postoperative period. Early recognition and prompt management are important.     

Surgical Placement of Totally Implantable Venous Access Device–An Institutional Experience

Objectives

To investigate the advantages and disadvantages of Totally implantable venous access devices (TIVAD) catheter in pediatric age group and also to review this subject in the recent literature and compare the results with the present results.

Methods

A total of 61 cases, 2 mo to 14 y old, were included in the index study. TIVADs were implanted in these patients for chemotherapy. The device was inserted under general anesthesia (GA) by open technique. All the children were followed up for a mean period of 7.2 mo.

Results

Out of 61 cases, 9 cases had complications; 3 requiring removal of the port due to infection and in one case the device had to be reinserted due to malpositioning, one had late wound dehiscence with exposed port chamber which needed operative correction and four had minor wound infections. There was no mortality. Complications like hemo or pneumothorax, arterial puncture, hematoma formation were very less with open technique of insertion of the port. Most patients and their parents were satisfied with TIVAD.

Conclusions

Thus, TIVAD can be a useful device for many chronic patients who need an IV access for multiple injections especially in pediatric age group.     

Association of Hirschsprung’s disease and anorectal malformation: a systematic review

Background

The association of Hirschsprung’s disease (HD) and anorectal malformation (ARM) is rare. The exact incidence of this association is not known but HD coexisting with ARM has been reported in 2.3 to 3.4 % of ARM cases. Most of the reported cases in the literature have been single case reports. The aim of this systematic review was to determine the incidence of HD associated with ARM and its relationship to other syndromes.

Methods

A systematic review of the literature was performed for the keywords “association of Hirschsprung’s disease and anorectal malformation”, “aganglionosis and anorectal malformation” as well as “congenital megacolon and anorectal malformation”. Resulting publications were reviewed for epidemiology, operative treatment and morbidity. Reference lists were screened for additional cases.

Results

A total of 38 articles reported 90 cases of HD coexisting with ARM from 1952 to 2013. Twenty eight articles reported 40 single case reports of this association. Ten articles reported 50 cases of HD in case series of 2,465 ARM patients, resulting in an incidence of 2 % of this association. Gender was reported in 63 cases, with 30 males (48 %) and 33 females (52 %). Associated syndromes were reported in 23 patients: Currarino syndrome in 11, Down syndrome in 8, Cat eye syndrome in 3 and Pallister-Hall syndrome in one case. Extent of aganglionosis was reported in 49 cases and included classical rectosigmoid disease in 36, long segment aganglionosis in 5, total colonic aganglionosis in 7 and total intestinal aganglionosis in one patient. In 35 % of the patients stoma was created in the aganglionotic region and failed to work. There was a median delay of 8 months for the diagnosis of HD from initial diagnosis of ARM. Various surgical techniques were employed for the pull-through operation for HD.

Conclusion

The review confirms that the recognition of HD is often delayed because of the initial diagnosis of ARM and the fact that the dysfunctional colostomy is usually proximal to the affected aganglionotic bowel. There is a high incidence of associated syndromes when HD coexists with ARM.     

Addressing the taboo of medical error through IGBOs: I got burnt once!

Background

An I got Burnt once (IGBO) is a near-miss or actual clinical event, related to patient safety, that leaves a lasting impact on the health professional (HP) involved. The purpose of this study was to collect and categorize IGBOs from a variety of pediatric HPs and to determine whether the individual's clinical practice was altered as a result.

Methods

Semistructured interviews involved recollection of an IGBO and subsequent changes in clinical practice. The IGBOs were classified into one of the seven Canadian Medical Education Directives for Specialists (CanMEDS) roles and outcome of the event.

Results

Of the 38 pediatric HPs approached (25 doctors and 13 female nurses), 35 recalled an IGBO. Most (74 %) were classified to the CanMEDS Medical Expert role (with subcategorization into diagnostics (37 %), treatment (34 %), and clinical management (31 %) followed by communicator (14 %) and collaborator (12 %) roles). Half (55 %) of the respondents considered the IGBO event to be potentially life threatening event to the patient, resulting in no harm (63 %), disability (14 %), and fatality in 17 % of the cases. Most respondents (92 %) stated that IGBOs affected their medical practice for months and sometimes years after the event.

Conclusions

Most practitioners can recall an IGBO in their clinical practice. IGBOs may be a potential source of medical risk avoidance and reduction strategies, and worthy of further investigation by “deep dives” or root cause analysis     

Was steckt hinter der Überweisungsdiagnose „z. B. Immundefekt“?

Background

The aim of this study was to establish the frequency and characteristic symptoms of primary immune defects among patients referred to a specialized clinical immunology outpatient unit.

Patients and methods

Between May 2010 and May 2012, 210 patients were referred for examination with the tentative diagnosis of “e.g. immune defect”.

Results

Two thirds of the patients had upper airway infection, failure to thrive, fever of unknown origin, abscess, or pneumonia. Every third patient had a migration background. An immune defect was diagnosed in 36 (17?%) patients. The most frequently diagnosed immune defect was antibody deficiency of variable origin. The time from onset of symptoms to diagnosis was 30.3 months.

Conclusion

Children with the above mentioned symptoms and signs should be referred to a specialist outpatient clinic for further investigation to identify a potential primary immune defect.     

Addressing the variation of post-surgical inpatient census with computer simulation

Objective

This study describes the development of a Discrete Event Simulation (DES) of a large pediatric perioperative department, and its use to compare the effectiveness of increasing the number of post-surgical inpatient beds vs. implementing a new discharge strategy on the proportion of patients admitted to the surgical unit to recover.

Materials and methods

A DES of the system was developed and simulated data were compared with 1 year of inpatient data to establish baseline validity. Ten years of simulated data generated by the baseline simulation (control) was compared to 10 years of simulated data generated by the simulation for the experimental scenarios. Outcome and validation measures include percentage of patients recovering in post-surgical beds vs. “off floor” in medical beds, and daily census of inpatient volumes.

Results

The proportion of patients admitted to the surgical inpatient unit rose from 79.0 % (95 % CI, 77.9–80.1 %) to 89.4 % (95 % CI, 88.7–90.0 %) in the discharge strategy scenario, and to 94.2 % (95 % CI, 93.5–95.0 %) in the additional bed scenario. The daily mean number of patients admitted to medical beds fell from 9.3 ± 5.9 (mean ± SD) to 4.9 ± 4.5 in the discharge scenario, and to 2.4 ± 3.2 in the additional bed scenario.

Discussion

Every hospital is tasked with placing the right patient in the right bed at the right time. Appropriately validated DES models can provide important insight into system dynamics. No significant variation was found between the baseline simulation and real-world data. This allows us to draw conclusions about the ramifications of changes to system capacity or discharge policy, thus meeting desired system performance measures.     

Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome

Purpose

Total colonic aganglionosis (TCA) is a severe form of Hirschsprung’s disease (HD), occurring in less than 10 % of the cases. It is a challenging surgical condition and outcomes of pull-through (PT) surgery are reported to be inferior to those in patients with recto-sigmoid HD. As even large centres only see a few patients with TCA, there is little information on the long-term outcome of patients after PT operation for TCA. The aim of this meta-analysis was to investigate the long-term clinical outcome in patients with TCA.

Methods

MEDLINE^® and EMBASE^® databases were searched for relevant articles that reported the outcomes of patients with TCA published between 1980 and 2011. The search terms were “Hirschsprung’s disease”, “Total colonic aganglionosis” AND “Outcome”. All published studies containing adequate clinical data for a mean follow-up period of not less than 4 years were included. Reference lists of retrieved articles were reviewed for additional cases. Detailed records of morbidity and mortality were extracted and analysed.

Results

This search yielded 225 articles reporting on outcomes in TCA. Of these, 189 were excluded for having too short a follow-up period, small or single case series, inadequate clinical data and duplicated patient groups. Ultimately, 36 articles from 37 centres containing useful clinical information on the outcomes of TCA in 969 patients were identified. There were 152 early deaths prior to PT (15.7 %). Of 817 survivors, 739 underwent PT. The mortality rate for TCA post-PT was 5.7 %. The most frequently reported post-operative complication was enterocolitis in 42 % of the cases. 17.5 % of patients underwent subsequent major surgery including redo PT, stoma reformation or other laparotomy. Long-term follow-up data were available in 396 patients. Satisfactory or normal bowel control was reported in 60 % of the patients. Soiling, faecal incontinence or other poor outcome was reported in 33.5 % of the cases and 6.5 % of the patients had undergone conversion to a permanent ileostomy for post-operative complications.

Conclusion

This meta-analysis reveals that a large number of patients with TCA have long-term problems with bowel control.     

Unexpected results of a nationwide, treatment-independent assessment of fecal incontinence in patients with anorectal anomalies

Purpose

To determine the anorectal function in patients with anorectal malformations (ARM) in order to facilitate patient counseling and follow-up.

Methods

Data were collected by the German network for urorectal malformations (CURE-Net) according to the International Krickenbeck consensus. Questionnaires on bowel function and a defecation protocol were completed by the families/patients. The clinical findings were assessed from the patients’ clinical records.

Results

Two hundred and ninety-seven patients with ARM were assessed, 175 patients gave complete data on continence, 52 of them were excluded due to mental retardation, age, and earlier type of pullthrough. Complete continence was found in 27 %, perineal fistula in 40 %, rectourethral/vesical in 10 %, vestibular in 24 %, cloaca in 0 %. Krickenbeck grade 1 soiling: 42 %, grade 2 and 3: 31 %. Forty-nine percent of the incontinent patients practiced bowel management, reaching continence in 19 %. The statement of constipation (67 %) was validated with the last clinical findings, showing coprostasis in 46 %, “Not suffering constipation” was confirmed in 61 % and falsified in 29 %.

Conclusion

ARM patients in Germany, as assessed by independent researchers, show a high rate of fecal incontinence and insufficiently treated constipation. Parents should be counseled accordingly and motivated to engage in consequent follow-up. Intensified efforts in the conservative treatment of constipation and fecal incontinence are crucial to improvement.     

Surgical treatment of non-cystic fibrosis bronchiectasis in Brazilian children

Purpose

To determine the clinical characteristics of patients submitted to surgical treatment for non-cystic fibrosis (CF) bronchiectasis, the indications for surgery, and the results obtained at a referral facility for pediatric thoracic surgery.

Methods

Between January 1998 and December 2009, we retrospectively reviewed the medical charts of 109 pediatric patients with non-CF bronchiectasis who underwent surgical treatment. These findings were subsequently analyzed by focusing on postoperative complications and long-term results.

Results

Of the 109 patients undergoing pulmonary resection, the mean age was 7.6 years (ranging from 1 to 15.5 y–o) with male predominance (59 %). The most common procedure was segmentectomy (43 %) followed by left lower lobectomy (38 %). Minor postoperative complications occurred in 36 % of the patients; the most common was transient atelectasis (26 %), followed by air leak (6 %), and postoperative pain (4 %). There was one death within the 30-day postoperative period, but it was unrelated to the procedure. Eighty-three children were followed after discharge, with a mean follow-up period of 667 days. Sixty-five (76 %) patients showed improvement of clinical symptoms after surgery.

Conclusions

Lung resection for the treatment of non-CF bronchiectasis in children is a safe procedure, with no life-treating morbidity and low mortality. This procedure also leads to significant improvements in symptoms and quality of life.     

Clinical characteristics and outcome of hepatocellular carcinoma in children and adolescents

Purpose

Hepatocellular carcinoma (HCC) rarely occurs in children and adolescents and has been reported to be highly hepatitis B related more than 10 years ago. However, after global vaccination for hepatitis B virus (HBV), the characteristics and outcome of pediatric HCC remain undefined.

Methods

Patients with HCC admitted from 2004 to 2010 were retrospectively reviewed in a large tertiary hospital.

Results

45 (1.97 %) pediatric HCC were diagnosed (age ≤18 years), with predominantly male patients (93.3 %). 32 (71.1 %) children were HBV positive, 30 of whom had vertical transmission from their mothers. HBV positivity was associated with liver cirrhosis and portal vein invasion, and thus compromised survival. Advanced disease prevented surgical resection due to large tumor size (>10 cm, 66.7 %), early metastasis (24.4 %), bilateral involvement (57.8 %) and portal vein invasion (46.7 %). The median survival for resectable, transarterial chemotherapy and embolization and untreated patients was 28.6, 4 and 5 months, respectively (p < 0.001). Patients with distal metastasis had significantly poorer survival rate than those without metastasis (p < 0.001).

Conclusion

Screening of children whose mothers are HBV carriers is important in early detection of pediatric HCC. HBV-associated HCC in pediatric patients, especially in endemic areas, should be detected earlier for more resectability and improvement of surgical prognosis.     

Chronic Kidney Disease (CKD): An Observational Study of Etiology,Severity and Burden of Comorbidities

Objectives

To study the etiology and burden of comorbidities across stages of chronic kidney disease (CKD).

Methods

Children, 2–16 y of age with CKD Stages II- IV were recruited over 12 mo. The etiology, clinical presentation and severity of complications were studied.

Results

Among 78 children [Stage II (n = 21), Stage III (n = 26), Stage IV (n = 31)], congenital anomalies of the kidney and urinary tract (CAKUT) was the commonest etiology and 28 were newly diagnosed in Stage III /IV. High prevalence of comorbidities was observed with growth retardation (65%), hypertension (59%), hyperphosphatemia (32%), vitamin D deficiency (92%), dyslipidemia (64%), left ventricular hypertrophy (45%) and hyperparathyroidism (56%). While most comorbidities correlated with the estimated glomerular filtration rate and severity of CKD, hypertension, vitamin D deficiency and cardiovascular morbidity were prevalent even in early stages.

Conclusions

CAKUT was the commonest cause of CKD. Late detection and high prevalence of comorbidities even in early stages of CKD were observed.

     

Needle core biopsy in the diagnosis of pediatric thyroid neoplasms: a single institution retrospective review

Purpose

Our institution routinely utilizes needle core biopsy (NCB), instead of fine needle aspiration, in the evaluation of pediatric thyroid nodules. This practice initially arose from limited cytopathology services in our hospital. Given the lack of information regarding the utility of NCB in diagnosing pediatric thyroid neoplasms, we set out to review our institution’s experience with this technique.

Methods

We performed a single institution retrospective chart review of all children who underwent thyroidectomy for primary thyroid pathology.

Results

Seventy-four patients, with a mean age of 12.9 ± 4.5 (SD) years, underwent partial or total thyroidectomy between 2002 and 2010. Seven of these patients had medically refractive hyperthyroidism. The remaining 67 patients had one or more thyroid nodules as identified by ultrasound. 24 (36 %) of these cases were malignant on final pathology. 14 (58 %) of the malignant cases were papillary thyroid carcinoma. 46 of the thyroid nodule cases underwent pre-operative NCB. Biopsy results for these patients were non-diagnostic in 6 (13 %), benign in 11 (24 %), atypical in 17 (37 %), and malignant in 12 (26 %). There were no complications arising from NCB. Sensitivity of NCB for diagnosing papillary carcinoma (PC) and follicular neoplasm was calculated at 0.88 (0.47–1.0, 95 % CI) and 0.84 (0.60–0.97, 95 % CI), respectively. Of the 28 patients not undergoing preoperative NCB, 12 underwent hemithyroidectomy, with one patient (8 %) requiring completion thyroidectomy for PC. Overall, the sensitivity of NCB in diagnosing PC and follicular thyroid neoplasms was 0.85 (0.55–0.99, 95 % CI), while the specificity was 0.63 (0.42–0.82, 95 % CI).

Conclusions

Needle core biopsy appears to have a low rate of associated complications, and its sensitivity for diagnosing PC and follicular neoplasm is comparable to what has been reported for fine needle aspiration biopsy in a similar patient population.     

Long-term results of transanal pull-through for Hirschsprung’s disease: a meta-analysis

Introduction

Several operative techniques have been developed for the treatment of Hirschsprung’s disease (HD) in the past decades. Since one-stage transanal pull-through (TAPT) was first performed in 1998, multiple studies have shown favourable short-and midterm results compared to other techniques with shorter operation length, shorter hospital stay and lower complication rates. The aim of this meta-analysis was to determine the longterm results following TAPT for HD.

Methods

A systematic literature search for relevant articles was performed in four databases using the following terms “Hirschsprung/Hirschsprung’s disease”, “aganglionosis”, “transanal”, “pullthrough/pull-through”, “longterm/long-term” “results”, “follow-up” and “outcome”. A meta-analysis was conducted for relevant articles for one-stage transanal pull-through for HD with a minimal follow-up of median 36 months regarding constipation, incontinence/soiling, enterocolitis and secondary operations. Odds ratio (OR) with 95 % confidence intervals (CI) were calculated.

Results

Six studies with 316 patients matched the set criteria and were included in this analysis. Overall 45 (14.2 %) patients had disturbances of bowel function (OR 0.05, 95 % CI 0.03–0.07, p < 0.00001). Of these, 24 (53.3 %) patients experienced constipation, 8 (17.8 %) incontinence/soiling and 13 (28.9 %) enterocolitis. 10 (3.2 %) patients developed complications requiring secondary surgery. Most patients had a daily defecation frequency of 1–3 bowel movements 3 years postoperatively, resembling the stooling patterns of healthy controls.

Conclusion

Nearly 15 % of all patients operated with TAPT for HD continue to experience persistent bowel symptoms with constipation as the main problem. Further studies on the long-term outcome of children operated with this technique for HD are necessary to evaluate stooling patterns, urinary and sexual function as well as general quality of life during adolescence and adulthood.